Rheumatoid anemia Masson, Charles
Joint, bone, spine : revue du rhumatisme,
03/2011, Volume:
78, Issue:
2
Journal Article
Peer reviewed
Abstract Rheumatoid anemia is a typical example of anemia of chronic disease. It differs from other forms of anemia, such as iron deficiency anemia or iatrogenic anemia. Rheumatoid anemia is ...normochromic, normocytic or, less often, microcytic, aregenerative, and accompanied with thrombocytosis. Serum transferrin levels are normal or low, transferrin saturation is decreased, serum ferritin levels are normal or high, the soluble transferrin receptor (sTfR) is not increased (a distinguishing feature with iron deficiency anemia), and the sTfR/log ferritin ratio is lower than 1. This review discusses the prevalence and impact of rheumatoid anemia based on a review of the literature. Iron metabolism, absorption, diffusion, storage, and use by the bone marrow are described using published data on transferrin, ferritin, and hepcidin. Hepcidin is now recognized as a key factor in rheumatoid anemia, in conjunction with the cytokine interleukin-6 (IL-6). Hepcidin is a hormone that lowers serum iron levels and regulates iron transport across membranes, preventing iron from exiting the enterocytes, macrophages, and hepatocytes. In addition, hepcidin inhibits intestinal iron absorption and iron release from macrophages and hepatocytes. The action of hepcidin is mediated by binding to the iron exporter ferroportin. Hepcidin expression in the liver is dependent on the protein hemojuvelin. Inflammation leads to increased hepcidin production via IL-6, whereas iron deficiency and factors associated with increased erythropoiesis (hypoxia, bleeding, hemolysis, dyserythropoiesis) suppress the production of hepcidin. Data from oncology studies and the effects of recombinant human IL-6 support a causal link between IL-6 production and the development of anemia in patients with chronic disease. IL-6 diminishes the proportion of nucleated erythroid cells in the bone marrow and lowers the serum iron level, and these abnormalities can be corrected by administering an IL-6 antagonist. IL-6 stimulates hepcidin gene transcription, most notably in the hepatocytes. Studies involving human hepatocyte exposure to a panel of cytokines showed that IL-6, but not TNFα or IL-1, induced the production of hepcidin mRNA. Recent data on hepcidin level variations in patients with rheumatoid arthritis are reviewed. Rheumatoid anemia is best corrected by ensuring optimal control of systemic disease activity. The role for iron supplementation (per os or intravenously) and erythropoietin in the treatment of rheumatoid anemia is discussed. Given the cascade of interactions linking IL-6, hepcidin, and anemia, IL-6 antagonists hold considerable promise for the management of rheumatoid anemia.
La pyomyosite, au-delà des tropiques Masson, Charles
Revue du rhumatisme monographies,
April 2022, 2022-04-00, Volume:
89, Issue:
2
Journal Article
Peer reviewed
La pyomyosite, infection bactérienne d’un (ou plusieurs) muscle squelettique du tronc, des membres, du petit bassin est le plus souvent due à un Staphylococcus aureus, exprimant la toxine leucocidine ...de Panton et Valentine. Décrite initialement comme une « pyomyosite tropicale », elle peut survenir sous tous les climats, avec une nette augmentation de son incidence, à la fois chez des enfants et des adultes. Elle s’installe progressivement, en trois stades anatomiques, aboutissant à l’abcédation. Des facteurs de risque existent mais sont inconstants. Elle peut se compliquer d’emboles ou d’un choc septique en fonction du retard dans le diagnostic, la prise en charge ou bien la fragilité du patient. La présence d’une arthrite septique de voisinage fait discuter l’origine de l’infection, pyomyosite primitive versus secondaire. L’imagerie efficace comporte l’échographie de la région douloureuse, le scanner et surtout l’IRM. Le germe est identifié assez rarement sur les hémocultures, possiblement sur la ponction sous échographie ou scanner du muscle infecté. D’autres germes tels que Staphylococcus aureus peuvent être responsables. Parfois, la pyomyosite est pluribactérienne. Une recherche PCR par l’ARN 16S ribosomal dans le matériel de prélèvement musculaire est proposée. L’atteinte monomusculaire avec syndrome inflammatoire fait discuter aussi une myosite focale ou un infarctus musculaire. L’antibiothérapie systématique est donnée pour une durée de trois à quatre semaines, focalisée initialement sur le Staphylococcus aureus, mais parfois également sur un bacille négatif en cas d’immunosuppression et adaptée ensuite. Une collection liquidienne abcédée peut être ponctionnée sous échographique ou scanner, et parfois si nécessaire au bloc opératoire.
Pyomyositis, a bacterial infection of one or more skeletal muscles of the trunk, limbs, or pelvis, is most often caused by Staphylococcus aureus, apparently expressing the Panton and Valentine leukocidin toxin. Initially described as a tropical disease, it can occur in all climates, with a clear increase in its incidence, both in children and adults. It progresses in three anatomical stages leading to an abscessed collection. Risk factors exist but are inconsistent. It may be complicated by septic emboli depending on the delay in diagnosis and therefore management or the frailty of the patient. The presence of septic arthritis in the vicinity leads to discussion of the origin of the infection, primary versus secondary pyomyositis. Effective imaging includes ultrasound of the painful area, CT-scan and especially MRI. The microroganism is identified quite rarely on blood cultures, possibly on puncture under ultrasound or CT-scan of the infected muscle. Other bacteria than Staphylococcus aureus can be responsible. Sometimes pyomyositis is multi-bacterial. A PCR research by 16S ribosomal RNA in the muscle sampling material is proposed. Single muscle involvement with inflammatory syndrome also leads to discussion of focal myositis or muscle infarction. Pyomyositis implies systematic antibiotic therapy given for a period of three to four weeks, focused initially on Staphylococcus aureus, but sometimes also on a negative bacillus in the case of immunosuppression, and adapted subsequently according to bacteriological results/evolution. An abscessed fluid collection can be drained under ultrasound or CT-scan, and sometimes if necessary in the operating room.
ObjectivesTo study the characteristics of B-cell non-Hodgkin’s lymphoma (NHL) or Hodgkin lymphoma complicating rheumatoid arthritis (RA) and to identify RA-related factors associated with their ...occurrence.MethodsA multicentre case–control study was performed in France. Cases were patients with RA fulfilling ACR-EULAR 2010 criteria in whom B-cell NHL or Hodgkin lymphoma developed after the diagnosis of RA. For each case, 2 controls were assigned at random from the ESPOIR cohort and were matched on age at lymphoma diagnosis (cases)/age at the 10-year follow-up visit in the cohort (controls). Case and control characteristics were compared to identify parameters associated with the occurrence of lymphoma.Results54 cases were included and matched to 108 controls. Lymphomas were mostly diffuse large B-cell lymphoma (DLBCL, n=27, 50.0%). On immunochemistry, 4 of 27 (14.8%) lymphoma cases were positive for Epstein-Barr virus. On univariate analysis, factors associated with the occurrence of lymphoma were male sex (OR 3.3, 95% CI 1.7 to 6.7), positivity for ACPA (OR 5.1, 95% CI 2.0 to 15.7) and rheumatoid factor (OR 3.9, 95% CI 1.6 to 12.2), and erosions on radiographs (OR 3.8, 95% CI 1.7 to 8.3) and DAS28 (OR 2.0, 95% CI 1.5 to 2.7), both at the time of matching. Methotrexate, TNF blockers and a number of previous biologics were not associated with the occurrence of lymphoma. On multivariable analysis, erosions and DAS28 remained significantly associated with increased risk of lymphoma.ConclusionLymphomas complicating RA are mostly DLBCL. Risk of lymphoma in patients with RA was increased with markers of disease activity and severity, which supports the paradigm of a continuum between autoimmunity and lymphomagenesis in RA.
Abstract Giant cell arteritis (GCA) affects middle-sized or large arteries in individuals over 50 years of age. GCA is characterized by a combination of focal inflammation responsible for arterial ...stenosis or occlusion and of systemic inflammation manifesting as polymyalgia rheumatica, a decline in general health, and inflammatory anemia. In addition to the typical involvement of the branches of the external carotid arteries, relatively common sites of involvement include the aorta, most notably in its thoracic segment, and the subclavian, axillary, brachial, vertebral, and femoral arteries. The treatment of GCA rests on daily glucocorticoid administration, which should be started on an emergency basis in patients with incipient visual impairments (diplopia or amaurosis fugax). The duration of glucocorticoid therapy is unpredictable and side effects are common. Initial megadose glucocorticoid therapy does not decrease subsequent glucocorticoid requirements. Glucocorticoid therapy regulates the Th17 pathway, which is involved in the prominent vascular and systemic manifestations; but not the Th1 pathway, which may underlie the chronic course of the disease (whereas aspirin, in addition to decreasing platelet aggregation, blocks the Th1 mediator interferon-gamma). Although GCA is classically described as resolving within 1 to 3 years, clinical practice often teaches otherwise. Many patients experience rebound abnormalities in laboratory tests and/or relapses, and some of them have recurrences after an apparently full recovery. Histological documentation is useful to confirm the diagnosis. The effect of methotrexate and TNFα antagonists is modest at best. A few patients have responded to tocilizumab, which suppresses IL-6, a key cytokine in GCA. Life expectancy in GCA patients is similar to that in same-age controls except for a slight excess in vascular mortality shortly after the diagnosis.
To determine geographical variation in the prevalence of rheumatoid arthritis (RA) and spondyloarthropathies (SpA) in France.
The survey sample was drawn from 7 areas of France. Households were ...randomly selected using the national telephone directory, and an individual within each household was randomly chosen by the next-birthday method. All cases of suspected RA and SpA were confirmed by the patient's rheumatologist or by clinical examination. Standardized estimates of prevalence were compared between regions and groups of regions.
In total 15,219 anonymous telephone numbers were selected. An average response rate of 64% led to a total of 9395 respondents included in the study. The highest regional rates of RA were observed in the south (range 0.59-0.66%), and the lowest in the north (range 0.14-0.24%), with a national rate of 0.31% (95% CI 0.18-0.48%). Regional heterogeneity was observed for SpA, with the highest rates in Bretagne (0.47%) and the Sud-Est (0.53%) and a national rate of 0.30% (95% CI 0.17-0.46%).
This study is the largest of its kind conducted in France. It shows inter-regional variations, mainly in RA, with a higher prevalence in the south of the country. The many potential reasons for the heterogeneity observed, including genetic and environmental factors, warrant further research.
Abstract Stickler syndrome is a group of rare genetic conditions (incidence, 1/7500 births) related to mutations in the collagen genes. Both the mutations and the clinical features vary widely across ...affected patients. The main manifestations are craniofacial birth defects, bone and joint symptoms, ocular abnormalities, and hearing loss. Stickler syndrome may be revealed at birth (25% of cases) by a combination of cleft palate, retrognathism, and micrognathism known as Pierre Robin sequence, which may cause neonatal respiratory problems. The ocular abnormalities include severe myopia, abnormalities of the vitreous, and a high risk of retinal detachment (60% of cases), which may cause blindness (4% of cases). Severe hearing loss with onset in early childhood may impair performance at school. Osteoarthritis (75% of patients) with onset before 30 years of age is a severe manifestation that causes chronic hip and low back pain and functional impairments. Joint replacement surgery is often required. The risk associated with multiple anesthesias is highest in patients with craniofacial defects. The bone status may deserve to be evaluated, as the combination of genetic abnormalities and physical impairments may promote bone loss. Clinicians should be cognizant of Stickler syndrome so that they can detect the disease in patients and their family members, prevent functional impairments, organize a multidisciplinary management strategy, and arrange for genetic counseling.
Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autosomal dominant inherited condition of periodic fever and pain. Most patients are of northern European descent. The ...attacks manifest as fever and pain in the joints, abdomen, muscles, skin, or eyes, with variations across patients. An acute-phase response occurs during the attacks. Patients with TRAPS are at risk for AA amyloidosis, the most common targets being the kidneys and liver. Soluble TNFRSF1A is usually low between the attacks and may be normal during the attacks, when TNF levels are high. TNFRSF1A is found in abnormally high numbers on leukocyte cell membranes. TRAPS is the first condition for which naturally occurring mutations in a TNF receptor were found; the mutations affect the soluble
TNFRSF1A gene in the 12p13 region. In some patients, the pathogenesis involves defective TNFRSF1A shedding from cell membranes in response to a given stimulus. Thus, TRAPS is a model for a novel pathogenic concept characterized by failure to shed a cytokine receptor. This review compares TRAPS to other inherited periodic febrile conditions, namely, familial Mediterranean fever, Muckle-Wells syndrome, cold urticaria, and hyper-IgD syndrome. The place of TRAPS relative to other intermittent systemic joint diseases is discussed. Colchicine neither relieves nor prevents the attacks, whereas oral glucocorticoid therapy is effective when used in dosages greater than 20 mg/day. The pathogenic hypothesis involving defective TNFRSF1A shedding suggests that medications targeting TNF may be effective in TRAPS.
The optimal management of pyogenic discitis is not agreed on. No randomized clinical trials of short-course or oral antibiotic regimens have been published to date. To shed light on this issue, we ...reviewed the management of patients admitted for pyogenic discitis to one of 12 networked rheumatology departments. In this cross-sectional observational study, each department included the first ten patients admitted starting in January 1997 for treatment of pyogenic discitis. One hundred ten patients met the inclusion criteria, 67 men and 43 women, with a mean age of 60.6 ± 13.7 years (range, 17–86 years). Mean time from symptom onset to diagnosis was 39.6 ± 39.8 days (range, 24 h–240 days). Blood cultures were positive in 47.3% of patients, and the percutaneous discal and vertebral biopsy in 63.6% of cases; these two investigations identified the causative organism in 79 cases (72.8%). Mean duration of the rheumatology department stay was 31.3 ± 14.1 days (range, 4–78 days). Antibiotics were given intravenously to 103 (93.6%) patients, for a mean of 25.5 ± 17.6 days (range, 4–124 days); duration of intravenous antibiotic therapy was longer than 4 weeks in 36.5% of patients. Only seven (6.4%) patients received primary oral antibiotics with no parenteral antibiotics. One hundred patients were given oral antibiotics at the same time as and after intravenous antibiotics, for a mean duration of 87.2 ± 43.6 days (range, 20–278 days); Bracing was used in 98 (89.1%) patients. Although antibiotic selection was rational and in agreement with current recommendations, wide differences were noted across centers regarding intravenous treatment duration, hospital stay duration, and total treatment duration.
Abstract Herpes zoster can be serious or incapacitating, particularly in patients whose immune system is compromised by a disease or treatment. Immunomodulating drugs can increase the risk of ...infection. Well-established risk factors include advanced age and glucocorticoid therapy. The data are somewhat conflicting for medications such as methotrexate, tofacitinib, TNFα antagonists (infliximab, adalimumab, etanercept, certolizumab, and golimumab), abatacept, tocilizumab, and rituximab. Nevertheless, the risk of herpes zoster is increased in patients taking biological agents, because of the underlying diseases and/or effects of the drugs. A live attenuated herpes zoster vaccine has been proven effective and safe in immunocompetent individuals. At present, however, it is not recommended for patients with immunodeficiencies, including those taking biological drugs, as no studies have assessed its risk/benefit ratio in this population. This situation may change in the near future, as recent data support the effectiveness and safety of the herpes zoster vaccine in patients who take biotherapies or have other causes of immunodeficiency. Alternative approaches designed to protect these patients from herpes zoster and its complications are also under evaluation. There is a need to define the indications of the herpes zoster vaccine in terms of the target population, timing, modalities, and frequency, according to the underlying chronic systemic disease, age group, varicella-zoster virus status, and exposure to therapeutic agents.
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